Diagnosis, Natural History, Management,tumor biology of Gastrinomas/PETs

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Gastrinomas are the most common symptomatic, malignant PET in patients with or without MEN1 and sufficient numbers of these patients have been entered into our protocols to allow systematic assessment. Studies are now underway, evaluating the natural history of these tumors including the a definition of factors determining prognosis and cause of death. Our studies have identified a cohort of 25% of patients in whom the gastrinomas have aggressive growth and 25% who have multiple endocrine neoplasia type 1 (MEN1). During this year information from our prospective studies was used to write a number of guideline and position papers with the North American Neuroendocrine Tumor Network and the European Neuroendocrine tumor network. In addition two surgical studies were completed including: a study of the effect of surgery in patients with negative preoperative imaging studies. This study demonstrated that tumors can be found in all patients, a proportion cured long-term; leading to the recommendation all of these patients should under exploration if no contraindications. A second study demonstrated for the first time that the presence of lymph node metastases only was associated with a poorer prognosis in patients with gastrinomas and that an increasing number of positive lymph nodes had proportional increased prognostic effect. Furthermore, a number of consensus conferences and position papers on classification/treatment of various disease aspects were published as well as a collaborative study of the need for preoperative neck imaging in patients with hyperparathyroidism and MEN1.